He natna hmuh tur awm lo tak hi i hriat duh bawk em? EGPA (Eosinophilic Granulomatosis With Polyangiitis) chungchang hi a awlsam zawngin han sawi ila!

Asthma i nei fo em? A nih loh leh allergy na tak nei i ni em? Tichuan ka sawi tur hi i tan a pawimawh hle mai thei. A chhan chu vawiin hian natna mak deuh leh hmuh tur awm lo tak, chutiang dinhmun nena inzawm fo thin chungchang kan sawi dawn a ni. He natna hi EGPA an ti a . Tun hma chuan Churg-Strauss syndrome tia hriat a ni. A hming hi a sei deuh niin a lang a nih chuan lungngai suh, awlsam takin i hrethiam ang u.

EGPA (Eosinophilic Granulomatosis With Polyangiitis) hi eng nge ni?

He hming sei tak hi a buaithlak deuh thei a, mahse a then hrang hrangah then ila, hrethiam ila. Tichuan a awlsam zawk hle mai.

1. Eosinophilic a ni

A awlsam zawngin eosinophilic tih awmzia chu i thisenah thisen var chi khat eosinophils an tih chu a tlangpui aia tam i nei tihna a ni. Hetiang hian han ngaihtuah teh, eosinophils hi kan taksaa vengtu te tak te ang mai an ni a, kan immune system an pui thin . Mahse EGPA vei mi pakhatah chuan eosinophils zat hi a chang chuan a pangngai aia a let hnih emaw, a aia tam emaw pawh a ni thei. Hei hian immune system hian overtime hna a thawk tihna a ni.

2. Granulomatosis natna (granulomatosis) a awm thin

Granulomatosis hi immune cells te hian taksa tissue ah hian inflammation, emaw swelling a awm chuan lump te te an siam thin a ni. Heng lumps te hi granulomas kan ti thin . Hei hi immune system hna pawh a ni. Harsatna hmun vel hual a, a darh lohna tur ven tum ang mai a ni.

3. Polyangiitis natna a ni

Polyangiitis hi i taksa chhunga thisen kalna kawng tam tak, a bik takin a te leh lian lo (poly tih hian tam tak tihna) te chu hun rei tak chhung a na a ni. Natna pawl lian zawk vasculitis an tih zinga mi a ni . Angiitis hi vasculitis tih hming dang a ni.

Chuvangin, heng zawng zawng hi dah khawm ila, EGPA hi natna hlauhawm tak a ni a, chu chu immune system hnathawh dan dik lo avanga lo awm a ni a, chu chu asthma emaw allergy emaw atanga intan a ni a, eosinophils a tipung a, granulomas a lo piang a, a tawpah chuan thisen kalna kawng a ti na a ni.

He EGPA natna hian engtin nge ka taksa a nghawng?

Thisen kalna kawng a inflammation chhunzawm zel, vasculitis , a awm chuan thisen kalna kawng bang chu a chak lo ta a ni. Hei hian balloon ang maiin an inzawm khawm thei a, an bulge thei bawk – chu chu aneurysm kan ti a ni. A nih loh leh, heng thisen kalna chak lote hi a puak darh thei a, taksaah thisen a chhuak thei bawk.

Thil dang pakhat chu inflammation hian thisen kalna kawng a ti na a, a hliam a, a chhungril a ti tawi thei bawk. Chuti a nih chuan eng nge thleng? Thisen kal dan a tichhe a, a chang chuan a tikhawlo vek bawk. Hei hian taksain a hnathawh that theihna tura oxygen leh nutrient a mamawh a hloh phah a ni.

Polyangiitis hian kan taksa pum puia thisen kalna kawng te te a nghawng avangin taksa khawi hmunah pawh symptoms a awm thei. Mahse, a nghawng tam ber chu thawkna lam , chu chu lung tihna a ni. Tin, kal (kidney), intestines, heart, leh nerve te a nghawng thei bawk.

EGPA natna lan chhuah dan chu engte nge ni?

He natna EGPA an tih hi a lo lang nghal lo. A lan chhuah dan chu zawi zawiin, kum tam tak chhung, stage engemaw zatah a lang chhuak thin. Chu stage te chu eng nge ni tih i en ang u.

Stage 1: A tir lama hmuh chhuah tlangpui

Hemi hun chhung hian hetiang hi i hre mai thei:

• Puitling atanga lo chhuak asthma: Asthma vei ngai lo pawhin asthma thar a nei thei a, a natna lan chhuah dan chu khuh, thawk hah, thawk harsa tih ang chi a ni.
• Hay fever: Hnar hah, hnar na leh hnar na a awm reng thei.
• Chronic Sinusitis: Sinus cavities inflammation avanga lu-a rit leh pressure awm.
• Nasal polyps: Hnar chhunga lo awm, cancer ni lo, hnar chhunga lo piang chhuak te tak te.
• Taksa natna tlangpui: I ruh a na ang maia i hriat, i taksa ruh a na.
• Khawsik, malaise, leh hahna: Natna leh chau tih mai mai.

Stage hnihna: Eosinophils pung zel chhinchhiahna

Natna a lo zual zel chuan chu eosinophils kan sawi tawhte chu taksa tissue hrang hrangah, a bik takin lungah an pung khawm tan thin. Hei hi eosinophilic infiltrates an ti a . Hei hian natna lan chhuahna thar a thlen thei a ni:

• Chhung na leh thawk harsa zual.
• Lung na tak tak.
• Skin rashes emaw spot emaw a awm thei.
• Digestive system symptoms: thil chi hrang hrang, pum na, ril\am.

Stage Pathumna: Vasculitis natna lan chhuah dan

Vasculitis , thisen kalna kawng a inflammation hi natna stage hnuhnung ber a ni tlangpui. Hei hian natna lan chhuahna na tak tak a thlen thei a, chungte chu:

• Internal bleeding chhinchhiahna: Ekvah thisen a awm a, khuh chhungah thisen a awm a, vun hnuaia hmun rawng danglam a awm bawk.
• Inflammation emaw, nerve tihchhiat emaw avanga symptoms: ke ruh natna, tingling, chak lohna, nerve na.
• Lung natna lan chhuah dan: thinlung thawk mumal lo, muhil, thawk hah, thawk hah (edema) .

Hei hi polyangiitis a ni.He natna hian kal (kidney) pawh a nghawng thei a, mahse a tam zawkah chuan symptom awm lovin a awm thin.

Engvangin nge he EGPA hi a lo chhuah? Eng nge a chhan?

Dik tak chuan he EGPA hi kan taksa hriselna (immune system) a thawk tha lo vang a ni . Ngaihtuah teh, hetah hian thil thleng hi kan taksa humhim tur sipaite, mahni taksa beih ang mai a ni. I thisen kalna kawng a inflammation te, a hmaa kan sawi tawh granulomas te lo awm te, eosinophils pung te – heng zawng zawng hi immune system a overactive vang leh a react dik loh vang a ni.

Mahse, zirchiangtute chuan hetiang thil hi a thlen chhan chiang takin an la hre thei lo.

Hun rei tak chhunga immune system inflammation nei natnate chu autoimmune diseases an ti \hin. Chutiang natna lan chhuah dan tlangpui chu taksa chhunga cell thenkhat laka antibodies siamin a bei thin hi a ni. Hei hi EGPA thlentu pakhat a ni thei.

Zirchiangtute chuan EGPA ang chi natna dang engemaw zat chu group khatah an dah a. An vai hian antibody bik ANCA tih an nei vek a ni. He pawl hi ANCA-associated vasculitis (AAV) an ti a. Mahse, EGPA vei zinga 40% chauhvin he ANCA antibody hi an nei a ni. Chumi awmzia chu a chhan awmchhun a ni lo tihna a ni.

Eosinophils pung hi vasculitis hmaa symptoms awm chhan hran niin a lang. Eosinophils hi tissue-ah a awm khawm chuan inflammation a siam a, tissue te a tichhe bawk. Hetianga eosinophils pung hi asthma leh allergy nen a inzawm tlat a ni. Asthma vei te hi midang aiin a let 34 in EGPA an vei tam zawk a ni.

EGPA avanga harsatna lian tham awm thei te chu engte nge ni?

He natna hi uluk taka enkawl a nih loh chuan i taksa peng leh taksa peng hrang hrangah chhiatna nasa tak a thlen thei a ni. Entirna tlemte chu hetiang hi a ni:

• Pleural effusion: Lung vel a tui awmkhawm.
• Pericardial effusion: Thinlung chhehvela tui awmkhawm.
• Peripheral neuropathy: Peripheral nerve tihchhiatna.
• Kidney natna rei tak awm thei.
• Thluak lama harsatna nei rei tak.
• Congestive heart failure a awm thin.

A pawimawh ber chu heng ang complication a lo thlen hma hian natna hi hriatchhuah leh enkawl hi a ni.

Engtin nge EGPA hi hmuhchhuah a nih?

EGPA hriatchhuah hi a harsa deuh thei a, a chhan chu hun hrang hrangah symptom hrang hrang i nei thei a ni. Nang leh i doctor in i dahkhawm vek theih nan hun rei tak a la ngai thei. Tin, EGPA symptoms hi natna dang tam tak symptoms nen a inang thei bawk.

Mahse, doctor chuan heng natna engemaw zat i neih chuan EGPA hi a ringhlel thei a ni:

• Asthma emaw hay fever emaw a neih tawhna.
• Thisen test-ah chuan eosinophil count a sang tih hmuhchhuah a ni.
• Imaging test-ah chuan eosinophilic infiltrates a awm tih hmuhchhuah a ni.
• Tissue biopsy-ah chuan granulomas leh/ emaw vasculitis a awm tih hmuhchhuah a ni.

He EGPA natna hi eng enkawlna nge ni? Hlauh tur a awm lo a ni lawm ni?

Ni e, he natna hi enkawlna tur EGPA tih hi a awm ngei ang. Lungngai suh! A bulpui ber chu natna hriat chian vat a, enkawl tan hi a ni.

A tlangpuiin, thil tih hmasak ber chu corticosteroids dose sang tak pek a ni a, chu chuan inflammation leh eosinophils tihziaawmna a ni. Heng damdawi te hian natna lan chhuah dan a control hian natna chu remission-ah a awm kan ti thin . Chu chu symptoms a bo tawh tihna a ni. Tichuan Doctor chuan damdawi dose chu a tihhniam zauh zauh a. Mi tam tak chuan he remission hi vawng reng turin corticosteroids hi a tlem berah an ei chhunzawm zel a ngai a ni.

A châng chuan corticosteroids chauh hi a tawk lo thei a, a nih loh leh side effects awm loh nan corticosteroids dose sang tak i ei chhunzawm a ngai thei bawk. Chutiang hunah chuan i doctor chuan damdawi dang a pe thei bawk.

Heng damdawi dang zingah hian immunosuppressants leh biologics te pawh a tel a ni . Mepolizumab hi US Food and Drug Administration (FDA) -in EGPA atana biologic a pawm hmasak ber a ni. Biologic dang , benralizumab pawhin tun hnaia clinical trial-ah result duhawm tak a nei bawk. He zirchianna thar hian EGPA vei tan enkawlna kawng thar a hawng a ni.

EGPA hmang hian nun chu eng ang nge ni ang? (Prognosis) tih a ni.

EGPA hi enkawl theih, mahse tihdam theih loh natna a ni. Mi tam zawk chuan damdawi hmangin an natna lan chhuah dan an control thei a, remission-ah an kal thei a ni. Chumi awmzia chu a symptoms te chu a bo vek tihna a ni. Mahse, enkawlna i chawlhsan chuan a natna lan chhuah dan chu a lo lang leh thei (relapse) . Chuvangin i doctor chuan i dam chhung zawngin i dinhmun a enkawl ang.

EGPA hmang hian rei tak dam theih a ni em?

Enkawlna tha tak i neih chuan EGPA hmang hian nun pangngai takin i hmang thei ang . Natna a zual zel chuan taksa peng hrang hrang (organ failure) ang chi harsatna hian i dam chhung a nghawng thei a ni. Mahse, enkawlna hian organ failure hi a titawp thei a, a tidanglam thei fo bawk. Enkawlna a awm chuan EGPA natna kum nga chhunga dam rei zat chu 80% aia tam a ni.

EGPA lo awm tur ven dan tur a awm em?

EGPA a lo awm chhan chiang taka kan la hriat loh avangin a ven dan tur kan hre lo. Mahse, a hmaa hriat chhuah leh enkawl chuan natna chu a zual zel loh nan a pui thei a ni . I natna lan chhuah dan uluk taka ngaihtuah a, i doctor hnena report hian EGPA natna khirh tak takte venna kawngah i pui thei ang.

EGPA nena kan awm chhung hian engtin nge ka in enkawl ang?

Hei hi zawhna pawimawh tak a ni. EGPA damdawi pek thin, a bik takin corticosteroid leh immunosuppressants te hian i immunity a ti tlem a ni . Chumi awmzia chu i damlo tam zawk tihna a ni a, i damloh chuan i dam leh theih nan hun rei zawk a ngai tihna a ni.

• Chuvangin, natna tlanglawn tak tak laka inven nan a hma aia fimkhur zawk a ngai a ni . Mipui tamna hmun leh damlo awmna hmuna kal hunah fimkhur hle ang che.
• Kut tha takin sil fo thin ang che.
• Ei leh in hrisel ei rawh.
• Mut nan i mamawh ang zat hun hmang rawh.
• Corticosteroids hi hun rei tak ei a nih chuan thisen sugar level sang (zunthlum ang chi), taksa rihna sang, ruh hring (osteoporosis ang chi), leh mood change te ang chi side effects a awm thei a ni Hengte hi i doctor nen sawiho la, enkawl dan tur zir rawh. Symptom thar i neih chuan doctor hnenah hrilh nghal rawh.

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Eosinophilic granulomatosis with polyangiitis (EGPA) hi vasculitis chi khat a ni a, a tlem hle bawk a, hriat a harsa hle bawk. I natna lan chhuahna chi hrang hrang, inzawm lo ang maia lang i nei thei. I doctor chuan an sawi tum chu a hrechhuak thei a, eng enkawlna nge i mamawh tih a rel theih nan hun rei tak a la ngai thei.

Thu lawmawm tak chu EGPA hi enkawlna hmangin a enkawl tha thei tih hi a ni . Mahse, i ni tin nunah inthlâk danglamna engemaw zat i siam a ngai ang. Mi tam takin an hriat ngai loh natna khirh tak nena nun chu a châng chuan mahni chauha awm thei a ni. Hetiang hunah hian EGPA vei midangte support network nena inzawmna siam hi a \angkai thei hle. Beiseina bansan ngai suh la, damdawi lam thurawn dik tak zawm la, nang pawhin nun \ha tak i nei thei ang.

` EGPA, Churg-Strauss syndrome, eosinophilic granulomatosis polyangiitis, thawhah, allergy, vasculitis, eosinophil te hi a awm thei a ni